Patient characteristics at diagnosis
| | HMIA (n = 31) | IBD (n = 30) |
|---|---|---|
| Age at symptom presentation | Prenatal: 11 (36%) | Prenatal: 0 |
| At birth: 15 (48%) | At birth: 7 (23%) | |
| After birth: 5 (16%) | After birth: 22 (77%) (missing data 1) | |
| Median age at genetic diagnosis (years) | 0.58 (0–29) | 2.91 (0–51) |
| Gender | M: 14 (45%) | M: 18 (60%) |
| F: 17 (55%) | F: 12 (40%) | |
| Liver derangement at diagnosis | 6 (20%) (missing data 2) | 6 (20%) (missing data 3) |
| Surgery at presentation | 28 (90%) | 3 (10%) |
| PN | 30/31 (97%) | 15/30 (50%) |
| Median age at PN start (years) | 0 (0–0.25) | 0.28 (0–1.30) |
| Indication to PN | Partial enteral tolerance: 2 (6%) | Partial enteral tolerance: 12 (80%) |
| No enteral tolerance: 27 (90%) | No enteral tolerance: 3 (20%) | |
| Surgery/transplant support: 1 (4%) | Surgery/transplant support: 0 |
| | HMIA ( | IBD ( |
|---|---|---|
| Age at symptom presentation | Prenatal: 11 (36%) | Prenatal: 0 |
| At birth: 15 (48%) | At birth: 7 (23%) | |
| After birth: 5 (16%) | After birth: 22 (77%) (missing data 1) | |
| Median age at genetic diagnosis (years) | 0.58 (0–29) | 2.91 (0–51) |
| Gender | M: 14 (45%) | M: 18 (60%) |
| F: 17 (55%) | F: 12 (40%) | |
| Liver derangement at diagnosis | 6 (20%) (missing data 2) | 6 (20%) (missing data 3) |
| Surgery at presentation | 28 (90%) | 3 (10%) |
| PN | 30/31 (97%) | 15/30 (50%) |
| Median age at PN start (years) | 0 (0–0.25) | 0.28 (0–1.30) |
| Indication to PN | Partial enteral tolerance: 2 (6%) | Partial enteral tolerance: 12 (80%) |
| No enteral tolerance: 27 (90%) | No enteral tolerance: 3 (20%) | |
| Surgery/transplant support: 1 (4%) | Surgery/transplant support: 0 |
M, male; F, female.
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